Serous choroidal detachment in chronic stage of Vogt-Koyanagi-Harada syndrome

Abstract

We report an atypical case of a 40-year-old woman with complete presentation of Vogt-Koyanagi-Harada Syndrome (VKH) who presented with unilateral serous choroidal detachment 17 years after the diagnosis. The patient complained of pain in the left eye, the ophthalmologic examination revealed visual acuity (VA) equal to hand motion; biomicroscopy revealed a granulomatous inflammatory reaction in the anterior chamber, tonometry was equal to 0 mmhg, and the fundoscopy was impracticable. Serous choroidal detachment was assessed by ocular ultrasonography. The therapeutic approach proposed for the patient consisted of prednisone 1mg / kg / day orally, dexamethasone 1mg / mL and atropine 1% eye drops. The evolution of the condition was satisfactory, with VA improvement to 20/40, absence of inflammatory reaction in anterior chamber, normalization of intraocular pressure and resolution of serous choroidal detachment in OS. We conclude that the chronic phase of VKH, although classically described as anterior uveitis, may have other presentations and the serous choroid detachment is a rare complication.

Keywords:
Panuveitis; Syndrome, Vogt-Koyanagi-Harada; Choroid diseases; Uveomeningoencephalitic syndrome; Case reports

Resumo

Relatamos um caso atípico de uma paciente de 40 anos com apresentação completa da Síndrome de Vogt-Koyanagi-Harada (SVKH) que após 17 anos do diagnóstico inicial evoluiu com descolamento seroso de coroide. A paciente procurou atendimento com queixa de dor em olho esquerdo (OE). O exame oftalmológico revelou acuidade visual (AV) igual a de movimento de mãos, à biomicroscopia foi observada reação inflamatória granulomatosa na câmara anterior, a tonometria foi igual a 0 mmhg, e a fundoscopia indevassável pela pouca midríase e turvação de meios em OE. O descolamento seroso de coroide foi avaliado através de ultrassonografia ocular. A abordagem terapeutica intituida para paciente consistiu em prednisona 1mg/kg/dia via oral, dexametasona 1mg/mL e atropina 1% colírios. A evolução do quadro foi satisfatória, com melhora da AV para 20/40, ausência de reação inflamatória em câmara anterior, normalização da pressão intraocular e resolução do descolamento seroso de coroide em OE. Concluimos que a fase crônica da SVKH, apesar da manifestação classicamente descrita ser uveíte anterior, pode ter outras apresentações e o descolamento seroso da coroide é uma rara complicação.

Descritores:
Panuveíte; Síndrome de Vogt-Koyanagi-Harada; Doenças da coroide; Síndrome uveomeningoencefalite; Relato de casos

Introduction

Vogt-Koyanagi-Harada Syndrome (VKHS) is an autoimmune disease directed at uveal melanocyte antigens. It is characterized by acute bilateral granulomatous panuveitis with exudative retinal detachments associated with involvement of the auditory, cutaneous and/or central nervous system.^(1-6)

The prevalence of the disease varies according to the population studied. However, it is generally more common in subjects with pigmented skin such as Asian and Hispanic, and in patients with genetic predisposition.^(1,4) Along with Behçet’s disease, it is one of the most prevalent causes of non-infectious uveitis in Brazil.⁽⁷⁾ VKH is more frequent in the adult population (between the third and fifth decades of life) affecting mainly women(5,8), and it is rare in Caucasians.⁽⁵⁾

The diagnosis of VKHS should base on the diagnostic criteria disclosed by the International Committee on Nomenclatures in 2000 and include: 1- absence of previous history of trauma or ocular surgery; 2- absence of evidence of concomitant ocular disease; 3 - early (with subretinal fluid focal areas or serous retinal detachment) or late (depigmentation, sunset fundus, Dalen-Fuchs depigmented chorioretinal scars, and migration or accumulation of pigment epithelium retina) bilateral ocular involvement; 4- history or presentation of auditory and/or neurological symptoms; 5- Skin changes with onset during or after neurological and ocular manifestations. It can be classified as complete when all criteria are present, incomplete when there is no criterion 4 or 5, and probable when there is absence of criteria 4 and 5.⁽⁴⁾

The classic clinical course of VKHS can be divided into 4 stages. The prodromal stage precedes the acute uveitic stage in some days, and simulates a viral infection. The acute uveitic stage manifests after a few days and has as clinical onset a bilateral diffuse panuveitis with serous detachments of the retina, hyperaemia of optical discs, and important reduction of visual acuity. Then, the convalescence stage is marked by signs of choroidal depigmentation, and later the recurrent chronic stage is evidenced by the presence of anterior uveitis.⁽⁹⁾ Although uncommon, subsequent involvement during recurrent chronic phase was reported based on clinical and subclinical evidence of imaging.^(5,6)

This report describes a case of serous choroidal detachment in the chronic stage of VKHS, a rare finding at this stage of the condition.

Case Report

ESO, 40 years old, female, brown, from Rio de Janeiro, diagnosed with VKHS in the complete form in 1998, characterized by previous tinnitus, bilateral granulomatous panuveitis and vitiligo (Figure 1), treated at the time with corticosteroid therapy at an immunosuppressive dose followed by slow withdrawal. The disease was worsened by secondary cataracts in both eyes (BE) and secondary glaucoma in the left eye (LE) treated surgically in 1999-2000 and 2002, respectively. Throughout the ophthalmological follow-up (1998-2015), she presented sparse recurrence episodes characterized by anterior uveitis and worsening of visual acuity, both treated with topical corticosteroids and mydriatics. She did not use any systemic immunosuppressants in the course of the disease.

Figure 1
Acromic spots in the menton and left malar region.

In 2015, the patient returned to the uveitis department complaining of eye pain and worsening of visual acuity in the LE. Ophthalmologic examination revealed visual acuity (VA) with correction equal to 20/40 in the right eye (RE) and hand movements in the LE. Biomicroscopy revealed old granulomatous ceramic precipitates in the lower third of the cornea with no signs of inflammation in the RE, and conjunctival hyperemia, granulomatous keratic precipitates in the lower third, cellularity 2+/4+, flare 1+/4+, topical intraocular lens, and trabeculectomy in the LE. Tonometry was equal to 9/0 mmHg. Fundoscopy revealed a regular stained optic disc with sharp edges, reduced foveal reflex, vascular tortuosity, and diffuse choroidosis in the RE, and indeburable due to poor mydriasis and media turbidity in the LE. Ophthalmologic ultrasound (USG) showed serous choroidal detachment in the LE (Figure 2) characterized by cupuliform, thick membranes with high reflectivity, low mobility and hyporeflective contents. Prednisone 1mg/kg/day orally, dexamethasone 1mg/mL eyedrops every hour with slow withdrawal, and atropine 1% eye drops every 12 hours were initiated.

Figure 2
Ultrasonography of the left eye (baseline) evidencing thick, cupuliform membranes with high reflectivity, low mobility, hyporeflective contents, corresponding to the serous detachment of choroid (red arrows). A. Cross section 3h; B. Cross section 6h; C. Cross section 9h; D. Cross section 12h.

Two weeks after the onset of symptoms the ophthalmologic examination revealed VA equal to 20/70 in the LE, ocular USG showed absence of membranes related to choroidal detachment (Figure 3), and tonometry was equal to 12mmHg. Topical and systemic steroids were gradualy withdrawn. One month later, VA was equal to 20/40 in the LE, and biomicroscopy showed 0.5+ cells in the LE, with treatment of the condition.

Figure 3
Ultrasonography of the left eye (follow up) evidencing absence of cupuliform membranes, showing treatment of serous choroidal detachment. A. Cross section 3h; B. Cross section 6h; C. Cross section 9h; D. Cross section 12h.

Discussion

VKHS occurs in four clinical stages. The prodromal phase shows vague and systemic symptoms5, 9, 10 including neurological and auditory manifestations such as headache, tinnitus, neck stiffness, and hearing loss.^(5,8,10,11)

The acute uveitic stage lasts several weeks and comprises bilateral granulomatous uveitis with vitreitis, white-yellow exudates on the retinal pigment epithelium (RPE), multifocal serous retinal detachment, and optic disc (OD) edema. The inflammation mainly involves the choroidal stroma with posterior involvement of RPE and external retina.^{(4,5,8,10,12,13)}

The uvetic stage is followed by the stage of convalescence in which tissue depigmentation is predominat.^(5,9) There is loss of the choroidal pigmentation responsible for the fundus of the eye in sunset glow (red-orange color due to depigmentation of the choroid present in 60-70% of the patients at this stage), peripapillary atrophy, limbic depigmentations (Sugiura’s Sign), vitiligo⁽¹⁴⁾, poliosis and alopecia.^(4,5,10,12)

In the present report, the patient presented manifestations related to the prodromal (tinnitus), uvetic (bilateral graulomatous panuveitis) and convalescence (vitiligo) stages, however without showing loss of choroidal pigmentation.

Some patients enter the chronic recurrent stage, mostly with recurrent episodes of anterior uveitis, ⁽⁹⁾ being commonly treated with topical medications (corticoid, cycloplegic and mydriatic).⁽¹⁰⁾ At this stage, there are also complications such as cataracts, glaucoma and choroidal neovascularization.^(4,8,14,15)

In the present study, besides cataract and secondary glaucoma, the patient showed recurrent anterior uveitis during disease follow-up.

Studies using indocyanine green (ICG) angiography have demonstrated the occurrence of subclinical choroidal inflammation concomitantly with anterior segment recurrence,^(6,16) bulging in the choroid⁽¹⁷⁾, and serous retinal detachment in the non-acute uveitis stage seen in optical coherence tomography (OCT).⁽⁵⁾ ICG can detect subclinical signs (dark dots and fuzzy vessels) in the chronic phase of posterior uveitis.⁽¹⁰⁾

The OCT guarantees better information on the retina, RPE, chorionic thickness, and qualitative and quantitative characteristics of the choroidal vasculature in panuveitis conditions.^(2,10) Choroid thickness was considered as a marker of VKH activity. However, this tool has not been widely used since several ocular and systemic factors interfere in this parameter, such as axial length, myopia, age and sex.⁽³⁾ It is not known which choroidal structures increase in volume in patients with VKH nor what happens to them in the progression or treatment of the disease.⁽²⁾ However, histopathological studies have shown that the choroid becomes thicker with lymphocytic infiltration.⁽¹³⁾ Therefore, the thickness of the choroid is known to be increased in the acute phase, progressively decreasing during disease progression (due to stroma healing, fibrosis, necrosis, melanocyte loss), and increases again in phases of exacerbation.⁽¹³⁾ Choroid lymphocytic infiltration is observed during the convalescence phase, demonstrating silent inflammation at this stage.⁽¹³⁾ Diffuse thickening of the choroid has already been described in the early and convalescent / chronic stages of VKH, and it has been suggested to represent a sign of disease activity.⁽¹⁷⁾

This case has the limitation of non-documentation of the posterior segment status with ICG or OCT during these recurrences.

Little is known about convalescence and chronic stages of VKH. Complete recurrences are rare after the end of the acute stage.⁽⁹⁾

Studies suggest that ocular involvement of VKHS occurs mainly in the posterior part of the uvea, with a predilection for the just-papillary region, and progressively decreases towards the equator, periphery of the choroid, and anterior uveal tissue. During the non-acute phase, relapse occurs as anterior uveitis directed to the remaining uveal melanocytes, since choroidal melanocytes were previously destroyed. However, supposedly quiescent cases are believed to have inflammatory activity in the unidentified posterior segment concomitant with the presence of cells in the anterior chamber. This characterizes relapse as a more extensive inflammatory involvement than believed, being inappropriately treated with topical corticoid.⁽¹⁰⁾

Once diagnosed, VKHS can be treated with various therapeutic regimens including corticosteroids, non-steroidal immunosuppressive therapy, biological response modifiers, and anti-VEGF (vascular endothelial growth factor)⁽⁵⁾, with the most effective therapy being the one with the least risk of long-term complications.⁽⁴⁾ In the case reported, the patient was treated with oral corticosteroid in an immunosuppressive dose, topical corticosteroid and mydriatic, and even after treatment of the serous detachment oral prednisone was used at a dose lower than 10mg/day.

Although classically described as an isolated anterior uveitis, the chronic phase of VKHS may have other presentations, and serous detachment of the choroid is a rare complication described in the literature by Sil et al.⁽¹¹⁾ and Yamoto et al.,⁽¹⁸⁾ but without specifying in which stage of the disease said finding occurred. There are no studies showing the prevalence of such a complication in VKHS.

In a similar way to the present report, Yamoto et al.⁽¹⁸⁾ described the case of a 59-year-old woman diagnosed with VKHS who developed choroidal annular detachment due to probable severe choroidal inflammation without retinal serous detachment associated. The patient was treated with systemic steroids, progressing successfully without recurrence after 4 years of follow-up. However, the authors did not relate choroidal detachment to any stage of VKHS. The treatment instituted for the patient of the present report was similar to that found in the literature and presented a favorable progression.

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Publication Dates

• Publication in this collection
  Jan-Feb 2019

History

• Received
  29 Jan 2018
• Accepted
  30 Mar 2018